阵发性睡眠性血红蛋白尿症血清α_2-巨球蛋白测定的临床意义  

Determination of Serum (?)2-Macroglobulin in Paroxysmal Nocturnal Hemoglobinuria and Its Clinical Significance

作　　者：徐开林[1] 鹿群先[1] 万美蓉[1] 赵洪国[2] 张春源[2] 翁维权[2] 

机构地区：[1]徐州医学院附属医院血液研究室,221002 [2]青岛医学院附属医院血液科

出　　处：《临床血液学杂志》1994年第1期14-16,共3页Journal of Clinical Hematology

摘　　要：测定了26例阵发性睡眠性血红蛋白尿症(PNH)患者和63例正常人血清α_2-巨球蛋白(α_2M)含量。结果显示,轻度溶血的PNH患者血清α_2M与正常对照比较差异无显著性;中度溶血者血清α_2M低于正常对照,P<0.05;重度溶血者α_2M显著低于正常对照,P<0.01。患者血清α_2M水平与血浆游离血红蛋白浓度及网织红细胞％呈负相关,P<0.05及0.01;与血红蛋白浓度呈正相关,P<0.05;与试管法凝血时间亦呈正相关,P<0.05。α_2M在2例合并血栓形成的PNH患者降低尤为显著。结果提示,测定血清α_2M可作为估计PNH患者红细胞破坏程度的一项指标;α_2M测定还有助于评价PNH时的高凝状态,当其显著降低时,要警惕血栓形成的可能。The levels of serum α2-macroglobulin (α2M) were determined in 63 normal subjects and 26 patients with paroxysmal nocturnal hemoglobinuria (PNH). In PNH Patients with severe hemolysis,the levels of serum α2M were markedly de- , creased as compared with normal controls. The levels of α2M were correlated with percentage of reticulocyte and concentrations of hemoglobin and plasma free hemoglobin. Determination of α2M might be useful in evaluating the extent of hemolysis in PNH. The levels of α2M were lower in 2 patients with thrombosis than the patients without thrombosis. The mechanism of decrease in α2M was discussed in this paper.

关 键 词：血红蛋白尿症 血清 巨球蛋白 

分 类 号：R556.640.6[医药卫生—血液循环系统疾病]